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Glossary

In this glossary you’ll find common terms that come up often when talking about CPAM, BPS and other congenital lung malformations. Use this as a quick reference guide in appointments or come back to it whenever you need to look something up or check your understanding.

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Medically reviewed and edited by Marisa Schwab, MD pediatric surgeon at Lucile Packard Children's Hospital at Stanford

Last edited: Sep 8 2025


A

Aorta – The main artery in the body. In some types of bronchopulmonary sequestrations (BPS) the mass gets its blood supply directly from a feeding vessel connected to the aorta.

Ascites – A build up of fluid around the baby's belly.

Asymptomatic – A baby shows no symptoms at birth and is able to breathe normally without support.

Atelectasis – A partial collapse of part of the lung

Attending – A very experienced senior doctor who is in charge of a patient's care. Attendings oversee residents and fellows, manage medical cases and make complex clinical decisions.

B

Betamethasone – A steroid used as a prenatal intervention to treat high risk cases of CPAM or BPS. The steroid can help to slow or shrink the growth of large lung masses. Two doses are given 24 hours apart.

BPS – Bronchopulmonary sequestration.

C

Cardiac effusion – A build up of fluid around the baby's heart.

CCAM – Congenital cystic adenoid malformation (the old term for CPAM).

Chest tube – A thin, flexible tube placed temporarily into the chest cavity to remove air, fluid, or blood from around the lungs after surgery.

CLE – Congenital lobar emphysema.

CLM – Congenital lung malformation.

Congenital – A condition that is present at birth.

CPAM – Congenital pulmonary lung malformation.

CPAP – A non-invasive type of breathing support that delivers a constant pressure of air and oxygen through a sealed cannula in the nose.

CT scan – Computed tomography scans use powerful x-rays to get very detailed images of structures inside the body. A CT scan is normally conducted within the first few months of life to help guide treatment and management options for the lung malformation. CT scans do expose the patient to radiation.

CVR – CPAM volume ratio. A common calculation used to estimate the size of the lung mass in relation to the size of the fetus or infant. A CVR of less than 1.6 is considered lower risk for complications.

Cyanosis – A discoloration of the skin, usually blue or gray, due to a lack of oxygen in the blood.

Cyst – An air or fluid-filled area of tissue.

E

Echocardiogram – An ultrasound carried out by a specialized technician to map the heart and blood flow.

EXIT procedure – Ex-utero intrapartum treatment is a highly specialized surgery where a baby is partially delivered via C-section but the umbilical cord keeps the baby attached to the mother's placenta (to receive oxygen and nutrients). The baby is then operated on before the umbilical cord is cut.

F

Feeder vessel – A blood vessel that supplies blood directly to a mass, lesion, or other area of tissue.

Fellow – A doctor who has graduated from medical school and completed a residency and is now doing further optional training in a speciality of medicine. Fellows are experienced, fully licensed doctors.

Fetal surgery – A very rare procedure where the baby is operated on while still in the womb.

H

High-flow air/nasal cannula – A non-invasive type of breathing support that delivers a high flow rate of air and oxygen through the nose.

Hybrid lesion – A combination of, usually, CPAM and BPS.

Hydrops – A rare but dangerous build up of fluid in at least two places that signals fetal distress.

I

Intubation – An invasive type of breathing support where a tube is inserted into the trachea (windpipe) so air and oxygen (and sometimes medication) can be delivered directly into the baby's lungs.

L

Lesion – A general term for abnormal tissue, often used interchangeably with "mass".

Lobe – The lungs are divided into distinct sections called lobes. There are three lobes on the right (upper, middle, lower) and two lobes on the left (upper, lower).

Lobectomy – A surgery to remove one lobe of the lung.

M

Macrocystic – A mass that is made up of one or more large cysts.

Malformation – Something that did not develop normally.

Mediastinal shift – The shifting of the heart and often also the trachea (windpipe) and esophagus from their normal central position, caused by a large lung mass or trapped air.

Microcystic – A mass that is made up of small cysts, usually many.

MFM – Maternal fetal medicine. A specialist prenatal doctor who works with pregnant women and unborn babies during complicated or high-risk pregnancies.

MRI – Magnetic resonance imaging uses a powerful magnetic field along with radio waves to map structures in the body in detail. MRIs produce more detailed images than ultrasounds. In a lung malformation, a fetal MRI may be performed after the abnormality is discovered to get a more precise image of the baby's lung mass. MRIs are not radiation and are safe during pregnancy.

N

Neonatologist – A specialist doctor who works with sick newborns and very young babies (typically under a year old).

NICU – Neonatal intensive care unit.

P

Pneumothorax – Sometimes called a collapsed lung. A condition where air leaks into the space between the lungs and the chest wall.

Polyhydramnios – A build up of amniotic fluid in the womb. Can indicate that the baby is not able to swallow amniotic fluid correctly, usually due to the lung malformation compressing the esophagus. Can be an early indicator of fetal stress.

PPB – Pleuropulmonary blastoma, a very rare form of childhood lung cancer that sometimes appears as a CPAM or BPS on early ultrasounds.

Persistent pulmonary hypertension of the newborn (PPHN) – A serious condition where the blood vessels in a newborn baby's lungs stay too tight after birth, preventing normal blood flow through the lungs. This makes it hard for the baby to get enough oxygen.

Pulmonary system – The respiratory system consisting of the lungs, airways, blood vessels and pulmonary arteries, and the diaphragm.

R

Radiologist – A specialist doctor who interprets images (like MRIs, X-rays, ultrasounds, CT scans) to diagnose diseases or conditions.

Resident – A doctor who has graduated from medical school and is training in a particular specialty of medicine, under supervision. Residents are fully licensed doctors.

Rounds – Daily (sometimes twice-daily) meetings where a medical team discusses a patient's ongoing care (usually at the bedside).

S

Symptomatic – A baby shows symptoms of not being able to breathe properly at birth and requires breathing support.

T

Tacypnea/tacypnic – Breathing faster than normal. A baby who is tachypneic is breathing very quickly, which can be a sign that they are working hard to get enough oxygen.

Thoracoamniotic shunt – A surgical procedure during pregnancy where a thin tube is inserted into the baby's CPAM to drain the cyst. This is very rare and only happens for high risk macrocystic cases.

Thoracoscopic/thoracoscopy – A minimally invasive ("keyhole") surgical procedure where the surgeon makes small incisions, and uses a camera and thin long instruments to do the surgery inside the chest.

X

X-ray – X-rays use electromagnetic radiation to map structures in the body. X-rays do not show lung malformations in detail and they often do not appear at all. X-rays are used once a baby is born, to see whether there are any obvious complications arising from the lung mass e.g. if the lungs have trapped air.